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Identification and targeting of an FGFR fusion in a pediatric thalamic "central oligodendroglioma".

ABSTRACT: Approximately 1-5% of pediatric intracranial tumors originate in the thalamus. While great strides have been made to identify consistent molecular markers in adult oligodendrogliomas, such as the 1p/19q co-deletion, it is widely recognized that pediatric oligodendrogliomas have a vastly different molecular make-up. While pediatric thalamic or "central oligodendrogliomas" are histologically similar to peripheral pediatric oligodendrogliomas, they are behaviorally distinct and likely represent a cohesive, but entirely different entity. We describe a case of a 10-year-old girl who was diagnosed with an anaplastic glioma with features consistent with the aggressive entity often diagnosed as central or thalamic oligodendroglioma. We performed whole-exome (paired tumor and germline DNA) and transcriptome (tumor RNA) sequencing, which demonstrated an FGFR3-PHGDH fusion. We describe this fusion and our rationale for pursuing personalized, targeted therapy for the patient's tumor that may potentially play a role in the treatment of similar cases.

SUBMITTER: Linzey JR 

PROVIDER: S-EPMC5871816 | biostudies-other | 2017

REPOSITORIES: biostudies-other

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Identification and targeting of an FGFR fusion in a pediatric thalamic "central oligodendroglioma".

Linzey Joseph R JR   Marini Bernard B   McFadden Kathryn K   Lorenzana Adonis A   Mody Rajen R   Robertson Patricia L PL   Koschmann Carl C  

NPJ precision oncology 20170907 1

Approximately 1-5% of pediatric intracranial tumors originate in the thalamus. While great strides have been made to identify consistent molecular markers in adult oligodendrogliomas, such as the 1p/19q co-deletion, it is widely recognized that pediatric oligodendrogliomas have a vastly different molecular make-up. While pediatric thalamic or "central oligodendrogliomas" are histologically similar to peripheral pediatric oligodendrogliomas, they are behaviorally distinct and likely represent a c  ...[more]